| [ Summary ] |
IgG4-related disease is a recently recognized clinicopathological entity characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis, and usually an elevated serum IgG4 concentration. IgG4-related tubulointerstitial nephritis and hydronephrosis associated with IgG4-related retroperitoneal fibrosis/periaortitis are major causes of renal failure. Although corticosteroids are quite effective, relapse is frequent with reduction or withdrawal of corticosteroids. In addition, the risk of malignancies is another problem associated with IgG4-related disease. |